Loading…
Seizure evolution and amino acid imbalances in murine succinate semialdehyde dehydrogenase (SSADH) deficiency
Mice with targeted deletion of the GABA catabolic enzyme succinic semialdehyde dehydrogenase (SSADH) manifest lethal tonic–clonic seizures, amenable to pharmacologic rescue, at 3–4 weeks of life. In the current report, we characterized amino acid profiles in SSADH −/− brain utilizing whole brain and...
Saved in:
| Published in: | Neurobiology of disease 2004-08, Vol.16 (3), p.556-562 |
|---|---|
| Main Authors: | , , , , , , |
| Format: | Article |
| Language: | English |
| Subjects: | |
| Citations: | Items that this one cites Items that cite this one |
| Online Access: | Get full text |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| cited_by | cdi_FETCH-LOGICAL-c446t-479282897e868daa900a22c78ddbfe87eea7eacdec80326ed899399b7e3987cb3 |
|---|---|
| cites | cdi_FETCH-LOGICAL-c446t-479282897e868daa900a22c78ddbfe87eea7eacdec80326ed899399b7e3987cb3 |
| container_end_page | 562 |
| container_issue | 3 |
| container_start_page | 556 |
| container_title | Neurobiology of disease |
| container_volume | 16 |
| creator | Gupta, M Polinsky, M Senephansiri, H Snead, O.C Jansen, E.E.W Jakobs, C Gibson, K.M |
| description | Mice with targeted deletion of the GABA catabolic enzyme succinic semialdehyde dehydrogenase (SSADH) manifest lethal tonic–clonic seizures, amenable to pharmacologic rescue, at 3–4 weeks of life. In the current report, we characterized amino acid profiles in SSADH
−/− brain utilizing whole brain and regional extracts (frontal and parietal cortex, hippocampus, and cerebellum) to develop hypotheses concerning epileptogenesis. Of 35 amino acids quantified, we found significant dysregulation in SSADH
−/− mice for 11 (GABA, glutamate, glutamine, alanine, aspartate, serine, taurine, cystathionine, methionine, homocarnosine, and arginine) as compared to age-matched littermates both before, and following, the period of generalized convulsive seizures and status epilepticus. Our results reveal imbalanced amino acid levels potentially involved in the transition from absence seizures to generalized convulsive seizures resulting in SSADH
−/− mice. We conclude that the SSADH
−/− mouse represents a unique epileptic model with the potential to reveal novel aspects of excitatory/inhibitory interactions in the genesis of seizures. |
| doi_str_mv | 10.1016/j.nbd.2004.04.008 |
| format | article |
| fullrecord | <record><control><sourceid>proquest_doaj_</sourceid><recordid>TN_cdi_doaj_primary_oai_doaj_org_article_f284c5118ba140f2b6b264e8591e3b00</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><els_id>S0969996104000981</els_id><doaj_id>oai_doaj_org_article_f284c5118ba140f2b6b264e8591e3b00</doaj_id><sourcerecordid>20537796</sourcerecordid><originalsourceid>FETCH-LOGICAL-c446t-479282897e868daa900a22c78ddbfe87eea7eacdec80326ed899399b7e3987cb3</originalsourceid><addsrcrecordid>eNqFkUuLFTEQhYMozp3RH-BGshJd9DVJd-eBq2F0nIEBF1fBXcijesylOxmT7oHrrzf3ge4UCioUXx0q5yD0ipI1JZS_366j9WtGSLfeF5FP0IoS1Teqb78_RSuiuGqU4vQMnZeyJYTSXonn6Iz2jDPGxQpNGwi_lgwYHtO4zCFFbKLHZgoxYeOCx2GyZjTRQcEh4mnJIQIui3Mhmrm-YApm9PBj5wEfWk73EE0B_Hazufx4865Oh-ACRLd7gZ4NZizw8tQv0LfrT1-vbpq7L59vry7vGtd1fG46oZhkUgmQXHpjFCGGMSek93YAKQCMAOM8OElaxsFLpVqlrIBWSeFse4Fuj7o-ma1-yGEyeaeTCfowSPlemzwHN4IemOxcT6m0hnZkYJZbxjuQvaLQWkKq1puj1kNOPxcos55CcTBWTyAtRXMuGOl491-Qkb4VQvEK0iPociolw_DnQkr0Pli91TVYvQ9W74vIuvP6JL7YCfzfjVOSFfhwBKD6-hgg63LwHHzI4Ob68fAP-d8B0LP2</addsrcrecordid><sourcetype>Open Website</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>20537796</pqid></control><display><type>article</type><title>Seizure evolution and amino acid imbalances in murine succinate semialdehyde dehydrogenase (SSADH) deficiency</title><source>ScienceDirect Journals</source><creator>Gupta, M ; Polinsky, M ; Senephansiri, H ; Snead, O.C ; Jansen, E.E.W ; Jakobs, C ; Gibson, K.M</creator><creatorcontrib>Gupta, M ; Polinsky, M ; Senephansiri, H ; Snead, O.C ; Jansen, E.E.W ; Jakobs, C ; Gibson, K.M</creatorcontrib><description>Mice with targeted deletion of the GABA catabolic enzyme succinic semialdehyde dehydrogenase (SSADH) manifest lethal tonic–clonic seizures, amenable to pharmacologic rescue, at 3–4 weeks of life. In the current report, we characterized amino acid profiles in SSADH
−/− brain utilizing whole brain and regional extracts (frontal and parietal cortex, hippocampus, and cerebellum) to develop hypotheses concerning epileptogenesis. Of 35 amino acids quantified, we found significant dysregulation in SSADH
−/− mice for 11 (GABA, glutamate, glutamine, alanine, aspartate, serine, taurine, cystathionine, methionine, homocarnosine, and arginine) as compared to age-matched littermates both before, and following, the period of generalized convulsive seizures and status epilepticus. Our results reveal imbalanced amino acid levels potentially involved in the transition from absence seizures to generalized convulsive seizures resulting in SSADH
−/− mice. We conclude that the SSADH
−/− mouse represents a unique epileptic model with the potential to reveal novel aspects of excitatory/inhibitory interactions in the genesis of seizures.</description><identifier>ISSN: 0969-9961</identifier><identifier>EISSN: 1095-953X</identifier><identifier>DOI: 10.1016/j.nbd.2004.04.008</identifier><identifier>PMID: 15262267</identifier><language>eng</language><publisher>United States: Elsevier Inc</publisher><subject>Aldehyde Oxidoreductases - deficiency ; Aldehyde Oxidoreductases - genetics ; Amino Acids - metabolism ; Animals ; Brain - metabolism ; Brain - physiopathology ; Disease Models, Animal ; Disinhibition ; Epilepsy ; Epilepsy - physiopathology ; GABA ; gamma-Aminobutyric Acid - metabolism ; Gamma-hydroxybutyric acid ; Glutamic Acid - metabolism ; Glutamine ; Glutamine - metabolism ; Knockout ; Mice ; Mice, Inbred C57BL ; Mice, Knockout ; Neural Inhibition ; Succinate-Semialdehyde Dehydrogenase ; Succinic semialdehyde dehydrogenase deficiency</subject><ispartof>Neurobiology of disease, 2004-08, Vol.16 (3), p.556-562</ispartof><rights>2004 Elsevier Inc.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c446t-479282897e868daa900a22c78ddbfe87eea7eacdec80326ed899399b7e3987cb3</citedby><cites>FETCH-LOGICAL-c446t-479282897e868daa900a22c78ddbfe87eea7eacdec80326ed899399b7e3987cb3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://www.sciencedirect.com/science/article/pii/S0969996104000981$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3549,27924,27925,45780</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/15262267$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Gupta, M</creatorcontrib><creatorcontrib>Polinsky, M</creatorcontrib><creatorcontrib>Senephansiri, H</creatorcontrib><creatorcontrib>Snead, O.C</creatorcontrib><creatorcontrib>Jansen, E.E.W</creatorcontrib><creatorcontrib>Jakobs, C</creatorcontrib><creatorcontrib>Gibson, K.M</creatorcontrib><title>Seizure evolution and amino acid imbalances in murine succinate semialdehyde dehydrogenase (SSADH) deficiency</title><title>Neurobiology of disease</title><addtitle>Neurobiol Dis</addtitle><description>Mice with targeted deletion of the GABA catabolic enzyme succinic semialdehyde dehydrogenase (SSADH) manifest lethal tonic–clonic seizures, amenable to pharmacologic rescue, at 3–4 weeks of life. In the current report, we characterized amino acid profiles in SSADH
−/− brain utilizing whole brain and regional extracts (frontal and parietal cortex, hippocampus, and cerebellum) to develop hypotheses concerning epileptogenesis. Of 35 amino acids quantified, we found significant dysregulation in SSADH
−/− mice for 11 (GABA, glutamate, glutamine, alanine, aspartate, serine, taurine, cystathionine, methionine, homocarnosine, and arginine) as compared to age-matched littermates both before, and following, the period of generalized convulsive seizures and status epilepticus. Our results reveal imbalanced amino acid levels potentially involved in the transition from absence seizures to generalized convulsive seizures resulting in SSADH
−/− mice. We conclude that the SSADH
−/− mouse represents a unique epileptic model with the potential to reveal novel aspects of excitatory/inhibitory interactions in the genesis of seizures.</description><subject>Aldehyde Oxidoreductases - deficiency</subject><subject>Aldehyde Oxidoreductases - genetics</subject><subject>Amino Acids - metabolism</subject><subject>Animals</subject><subject>Brain - metabolism</subject><subject>Brain - physiopathology</subject><subject>Disease Models, Animal</subject><subject>Disinhibition</subject><subject>Epilepsy</subject><subject>Epilepsy - physiopathology</subject><subject>GABA</subject><subject>gamma-Aminobutyric Acid - metabolism</subject><subject>Gamma-hydroxybutyric acid</subject><subject>Glutamic Acid - metabolism</subject><subject>Glutamine</subject><subject>Glutamine - metabolism</subject><subject>Knockout</subject><subject>Mice</subject><subject>Mice, Inbred C57BL</subject><subject>Mice, Knockout</subject><subject>Neural Inhibition</subject><subject>Succinate-Semialdehyde Dehydrogenase</subject><subject>Succinic semialdehyde dehydrogenase deficiency</subject><issn>0969-9961</issn><issn>1095-953X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2004</creationdate><recordtype>article</recordtype><sourceid>DOA</sourceid><recordid>eNqFkUuLFTEQhYMozp3RH-BGshJd9DVJd-eBq2F0nIEBF1fBXcijesylOxmT7oHrrzf3ge4UCioUXx0q5yD0ipI1JZS_366j9WtGSLfeF5FP0IoS1Teqb78_RSuiuGqU4vQMnZeyJYTSXonn6Iz2jDPGxQpNGwi_lgwYHtO4zCFFbKLHZgoxYeOCx2GyZjTRQcEh4mnJIQIui3Mhmrm-YApm9PBj5wEfWk73EE0B_Hazufx4865Oh-ACRLd7gZ4NZizw8tQv0LfrT1-vbpq7L59vry7vGtd1fG46oZhkUgmQXHpjFCGGMSek93YAKQCMAOM8OElaxsFLpVqlrIBWSeFse4Fuj7o-ma1-yGEyeaeTCfowSPlemzwHN4IemOxcT6m0hnZkYJZbxjuQvaLQWkKq1puj1kNOPxcos55CcTBWTyAtRXMuGOl491-Qkb4VQvEK0iPociolw_DnQkr0Pli91TVYvQ9W74vIuvP6JL7YCfzfjVOSFfhwBKD6-hgg63LwHHzI4Ob68fAP-d8B0LP2</recordid><startdate>20040801</startdate><enddate>20040801</enddate><creator>Gupta, M</creator><creator>Polinsky, M</creator><creator>Senephansiri, H</creator><creator>Snead, O.C</creator><creator>Jansen, E.E.W</creator><creator>Jakobs, C</creator><creator>Gibson, K.M</creator><general>Elsevier Inc</general><general>Elsevier</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7TK</scope><scope>7X8</scope><scope>DOA</scope></search><sort><creationdate>20040801</creationdate><title>Seizure evolution and amino acid imbalances in murine succinate semialdehyde dehydrogenase (SSADH) deficiency</title><author>Gupta, M ; Polinsky, M ; Senephansiri, H ; Snead, O.C ; Jansen, E.E.W ; Jakobs, C ; Gibson, K.M</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c446t-479282897e868daa900a22c78ddbfe87eea7eacdec80326ed899399b7e3987cb3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2004</creationdate><topic>Aldehyde Oxidoreductases - deficiency</topic><topic>Aldehyde Oxidoreductases - genetics</topic><topic>Amino Acids - metabolism</topic><topic>Animals</topic><topic>Brain - metabolism</topic><topic>Brain - physiopathology</topic><topic>Disease Models, Animal</topic><topic>Disinhibition</topic><topic>Epilepsy</topic><topic>Epilepsy - physiopathology</topic><topic>GABA</topic><topic>gamma-Aminobutyric Acid - metabolism</topic><topic>Gamma-hydroxybutyric acid</topic><topic>Glutamic Acid - metabolism</topic><topic>Glutamine</topic><topic>Glutamine - metabolism</topic><topic>Knockout</topic><topic>Mice</topic><topic>Mice, Inbred C57BL</topic><topic>Mice, Knockout</topic><topic>Neural Inhibition</topic><topic>Succinate-Semialdehyde Dehydrogenase</topic><topic>Succinic semialdehyde dehydrogenase deficiency</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Gupta, M</creatorcontrib><creatorcontrib>Polinsky, M</creatorcontrib><creatorcontrib>Senephansiri, H</creatorcontrib><creatorcontrib>Snead, O.C</creatorcontrib><creatorcontrib>Jansen, E.E.W</creatorcontrib><creatorcontrib>Jakobs, C</creatorcontrib><creatorcontrib>Gibson, K.M</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Neurosciences Abstracts</collection><collection>MEDLINE - Academic</collection><collection>Open Access: DOAJ - Directory of Open Access Journals</collection><jtitle>Neurobiology of disease</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Gupta, M</au><au>Polinsky, M</au><au>Senephansiri, H</au><au>Snead, O.C</au><au>Jansen, E.E.W</au><au>Jakobs, C</au><au>Gibson, K.M</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Seizure evolution and amino acid imbalances in murine succinate semialdehyde dehydrogenase (SSADH) deficiency</atitle><jtitle>Neurobiology of disease</jtitle><addtitle>Neurobiol Dis</addtitle><date>2004-08-01</date><risdate>2004</risdate><volume>16</volume><issue>3</issue><spage>556</spage><epage>562</epage><pages>556-562</pages><issn>0969-9961</issn><eissn>1095-953X</eissn><abstract>Mice with targeted deletion of the GABA catabolic enzyme succinic semialdehyde dehydrogenase (SSADH) manifest lethal tonic–clonic seizures, amenable to pharmacologic rescue, at 3–4 weeks of life. In the current report, we characterized amino acid profiles in SSADH
−/− brain utilizing whole brain and regional extracts (frontal and parietal cortex, hippocampus, and cerebellum) to develop hypotheses concerning epileptogenesis. Of 35 amino acids quantified, we found significant dysregulation in SSADH
−/− mice for 11 (GABA, glutamate, glutamine, alanine, aspartate, serine, taurine, cystathionine, methionine, homocarnosine, and arginine) as compared to age-matched littermates both before, and following, the period of generalized convulsive seizures and status epilepticus. Our results reveal imbalanced amino acid levels potentially involved in the transition from absence seizures to generalized convulsive seizures resulting in SSADH
−/− mice. We conclude that the SSADH
−/− mouse represents a unique epileptic model with the potential to reveal novel aspects of excitatory/inhibitory interactions in the genesis of seizures.</abstract><cop>United States</cop><pub>Elsevier Inc</pub><pmid>15262267</pmid><doi>10.1016/j.nbd.2004.04.008</doi><tpages>7</tpages><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0969-9961 |
| ispartof | Neurobiology of disease, 2004-08, Vol.16 (3), p.556-562 |
| issn | 0969-9961 1095-953X |
| language | eng |
| recordid | cdi_doaj_primary_oai_doaj_org_article_f284c5118ba140f2b6b264e8591e3b00 |
| source | ScienceDirect Journals |
| subjects | Aldehyde Oxidoreductases - deficiency Aldehyde Oxidoreductases - genetics Amino Acids - metabolism Animals Brain - metabolism Brain - physiopathology Disease Models, Animal Disinhibition Epilepsy Epilepsy - physiopathology GABA gamma-Aminobutyric Acid - metabolism Gamma-hydroxybutyric acid Glutamic Acid - metabolism Glutamine Glutamine - metabolism Knockout Mice Mice, Inbred C57BL Mice, Knockout Neural Inhibition Succinate-Semialdehyde Dehydrogenase Succinic semialdehyde dehydrogenase deficiency |
| title | Seizure evolution and amino acid imbalances in murine succinate semialdehyde dehydrogenase (SSADH) deficiency |
| url | http://sfxeu10.hosted.exlibrisgroup.com/loughborough?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-08T03%3A45%3A59IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_doaj_&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Seizure%20evolution%20and%20amino%20acid%20imbalances%20in%20murine%20succinate%20semialdehyde%20dehydrogenase%20(SSADH)%20deficiency&rft.jtitle=Neurobiology%20of%20disease&rft.au=Gupta,%20M&rft.date=2004-08-01&rft.volume=16&rft.issue=3&rft.spage=556&rft.epage=562&rft.pages=556-562&rft.issn=0969-9961&rft.eissn=1095-953X&rft_id=info:doi/10.1016/j.nbd.2004.04.008&rft_dat=%3Cproquest_doaj_%3E20537796%3C/proquest_doaj_%3E%3Cgrp_id%3Ecdi_FETCH-LOGICAL-c446t-479282897e868daa900a22c78ddbfe87eea7eacdec80326ed899399b7e3987cb3%3C/grp_id%3E%3Coa%3E%3C/oa%3E%3Curl%3E%3C/url%3E&rft_id=info:oai/&rft_pqid=20537796&rft_id=info:pmid/15262267&rfr_iscdi=true |